Clinical profile of arrhythmogenic right ventricular cardiomyopathy in Chinese patients

Objective: To study the clinical profile of Chinese patients with arrhythmo genic right ventricular cardiomyopathy (ARVC). Patients: Chinese patients w ho fulfilled the diagnostic criteria of ARVC proposed by the Task Force of the European Society of Cardiology and of the scientific council on cardiom yopathy of the International Society and Federation of Cardiology were recr uited for analysis. Methods: Clinical data of patients with ARVC including age, sex, family history, presenting symptoms, electrocardiograph (ECG), ec hocardiography, cardiac catheterization, magnetic resonance imaging (MRI), electrophysiology study (EPS) and therapeutic intervention were analyzed. R esults: Eleven patients (seven males) were diagnosed with ARVC. Mean age at clinical presentation was 42.6 +/- 14.8 years. Two patients (18.1%) had po sitive family history of ARVC or premature sudden cardiac death. The common est presenting symptoms were palpitation (73%) and dizziness (46%). Spontan eous ventricular tachycardia (VT) was the presenting arrhythmia in 54% and 1 (9%) with ventricular fibrillation and cardiac arrest. Seven patients (64 %) had the ECG abnormality as defined by the Task Force. Echocardiography s howed right ventricular (RV) dilatation in five patients (46%) and all pati ents had normal left ventricular function. Nine patients (90%) had RV wall thinning or fibrofatty replacement on MRI examination. Inducible monomorphi c VT was detected in four out of nine patients at EPS. All eight patients h ad normal coronary arteries and left ventriculogram. but RV dilatation and global hypokinesia was seen in three patients. Implantable cardioverter def ibrillators were implanted in five patients and two of them had shocks deli vered during the follow-up period. Conclusion: In this study, familial inci dence of premature sudden death in patients with ARVC appears to be low and left ventricular involvement in affected individuals is uncommon. MRI is s till the best investigation for ARVC. (C) 2001 Elsevier Science Ireland Ltd . All rights reserved.